J Pathol Transl Med.  2017 May;51(3):255-263. 10.4132/jptm.2017.01.12.

Clinicopathological Study of 18 Cases of Inflammatory Myofibroblastic Tumors with Reference to ALK-1 Expression: 5-Year Experience in a Tertiary Care Center

• Telugu RB
• Prabhu AJ
• Kalappurayil NB
• Mathai J
• Gnanamuthu BR
• Manipadam

• KMID: 2392593
• DOI: http://doi.org/10.4132/jptm.2017.01.12

Abstract

BACKGROUND
Inflammatory myofibroblastic tumor is a histopathologically distinctive neoplasm of children and young adults. According to World Health Organization (WHO) classification, inflammatory myofibroblastic tumor is an intermediate-grade tumor, with potential for recurrence and rare metastasis. There are no definite histopathologic, molecular, or cytogenetic features to predict malignant transformation, recurrence, or metastasis.
METHODS
A 5-year retrospective study of histopathologically diagnosed inflammatory myofibroblastic tumors of various anatomic sites was conducted to correlate anaplastic lymphoma kinase-1 (ALK-1) expression with histological atypia, multicentric origin of tumor, recurrence, and metastasis. Clinical details of all the cases were noted from the clinical work station. Immunohistochemical stains for ALK-1 and other antibodies were performed. Statistical analysis was done using Fisher exact test.
RESULTS
A total of 18 cases of inflammatory myofibroblastic tumors were found during the study period, of which 14 were classical. The female-male ratio was 1:1 and the mean age was 23.8 years. Histologically atypical (four cases) and multifocal tumors (three cases, multicentric in origin) were noted. Recurrence was noted in 30% of ALK-1 positive and 37.5% of ALK-1 negative cases, whereas metastasis to the lung, liver, and pelvic bone was noted in the ALK-1 positive group only.
CONCLUSIONS
Overall, ALK-1 protein was expressed in 55.6% of inflammatory myofibroblastic tumors. There was no statistically significant correlation between ALK-1 expression, tumor type, recurrence and metastasis. However, ALK-1 immunohistochemistry is a useful diagnostic aid in the appropriate clinical and histomorphologic context.

Keyword

ALK protein; Atypia; Prognostic marker; Recurrence; Neoplasms

MeSH Terms

Antibodies
Child
Classification
Coloring Agents
Cytogenetics
Humans
Immunohistochemistry
Liver
Lung
Lymphoma
Myofibroblasts*
Neoplasm Metastasis
Pelvic Bones
Recurrence
Retrospective Studies
Tertiary Care Centers*
Tertiary Healthcare*
World Health Organization
Young Adult
Antibodies
Coloring Agents

Figure

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  Fig. 1. Lobectomy specimen with a circumscribed nodular grey-white lesion with focal hemorrhage.

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  Fig. 2. (A, B) Classical inflammatory myofibroblastic tumor with bland spindle cell proliferation and pronounced inflammatory infiltrate.

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  Fig. 3. Diffuse (A) and focal (B) cytoplasmic immunohistochemical stains of anaplastic lymphoma kinase-1.

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  Fig. 4. Epithelioid inflammatory myofibroblastic sarcoma with increased cellularity, cellular atypia with large epithelioid like cells, multinucleated or anaplastic giant cells and atypical mitosis (A); perinuclear accentuation of anaplastic lymphoma kinase-1 expression (B).

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  Fig. 5. Inflammatory myofibroblastic tumor with increased cellularity, cellular atypia with large atypical spindle shaped cells (A) and focal atypical epithelioid or ganglion-like cells with hyperchromasia (B); negative immunoreactivity of anaplastic lymphoma kinase-1 (C).

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  Fig. 6. Atypical inflammatory myofibroblastic tumor with increased cellularity, cellular atypia with large atypical spindle shaped cells and hyperchromasia (A); negative immunoreactivity of anaplastic lymphoma kinase-1 (B).

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